Girtovitis Fotios I, Ntaios George, Papadopoulos Athanasios, Ioannidis Georgios, Makris Pantelis E
First Propedeutic Department of Internal Medicine, AHEPA Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Acta Haematol. 2007;118(2):117-22. doi: 10.1159/000107653. Epub 2008 Aug 27.
Hemorrhagic tendency in patients with myelodysplastic syndrome (MDS) is mainly attributed to thrombocytopenia. However, platelet function in these patients has not been thoroughly investigated.
The aim of our study is to evaluate platelet function in patients with primary MDS.
Platelet function was studied with aggregometry in response to ristocetin, collagen, ADP and adrenaline in 26 MDS patients and 15 healthy individuals.
Aggregation was defective in 21 patients (80.7%). Adrenaline was the agonist with the most profound defect (45.9%), followed by ADP (58.7%), whereas aggregation with ristocetin and collagen was at the borderline. Abnormal aggregation to all four agonists was detected in 6 patients (23%). On the contrary, aggregation results were normal in only 5 patients (19.2%). RAEB-t (refractory anemia with excess blasts in transformation) patients were most seriously affected.
MDS patients have impaired platelet aggregation in response to one or more stimulants. Platelet aggregation was not statistically different between MDS patients at early stages of the disease (<12 months) and those at later stages (>12 months). Defective platelet aggregation is strongly related to MDS of worse prognosis. None of our patients was detected to have hyperfunctional platelets, defined as platelets aggregating spontaneously. Functional defects in MDS do not elicit hemorrhagic tendency.
骨髓增生异常综合征(MDS)患者的出血倾向主要归因于血小板减少。然而,这些患者的血小板功能尚未得到充分研究。
我们研究的目的是评估原发性MDS患者的血小板功能。
采用血小板聚集试验研究了26例MDS患者和15名健康个体对瑞斯托霉素、胶原、二磷酸腺苷(ADP)和肾上腺素的血小板功能。
21例患者(80.7%)出现聚集功能缺陷。肾上腺素是缺陷最严重的激动剂(45.9%),其次是ADP(58.7%),而瑞斯托霉素和胶原诱导的聚集处于临界水平。6例患者(23%)对所有四种激动剂的聚集均异常。相反,只有5例患者(19.2%)的聚集结果正常。转化中的难治性贫血伴原始细胞增多(RAEB-t)患者受影响最严重。
MDS患者对一种或多种刺激的血小板聚集功能受损。疾病早期(<12个月)和晚期(>12个月)的MDS患者血小板聚集无统计学差异。血小板聚集缺陷与预后较差的MDS密切相关。我们的患者均未检测到血小板功能亢进,即血小板自发聚集。MDS中的功能缺陷不会引发出血倾向。